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Pathophysiology of childhood polycystic kidney diseases: new insights into disease-specific therapy.

Sweeney WE Jr, Avner ED.

Pediatr Res. 2014 Jan;75(1-2):148-57. doi: 10.1038/pr.2013.191. Epub 2013 Oct 31. Review.


Smac-mimetic-induced epithelial cell death reduces the growth of renal cysts.

Fan LX, Zhou X, Sweeney WE Jr, Wallace DP, Avner ED, Grantham JJ, Li X.

J Am Soc Nephrol. 2013 Dec;24(12):2010-22. doi: 10.1681/ASN.2013020176. Epub 2013 Aug 29.


Sirtuin 1 inhibition delays cyst formation in autosomal-dominant polycystic kidney disease.

Zhou X, Fan LX, Sweeney WE Jr, Denu JM, Avner ED, Li X.

J Clin Invest. 2013 Jul;123(7):3084-98. doi: 10.1172/JCI64401. Epub 2013 Jun 17.


New approaches to the autosomal recessive polycystic kidney disease patient with dual kidney-liver complications.

Telega G, Cronin D, Avner ED.

Pediatr Transplant. 2013 Jun;17(4):328-35. doi: 10.1111/petr.12076. Epub 2013 Apr 17. Review.


G-protein signaling modulator 1 deficiency accelerates cystic disease in an orthologous mouse model of autosomal dominant polycystic kidney disease.

Kwon M, Pavlov TS, Nozu K, Rasmussen SA, Ilatovskaya DV, Lerch-Gaggl A, North LM, Kim H, Qian F, Sweeney WE Jr, Avner ED, Blumer JB, Staruschenko A, Park F.

Proc Natl Acad Sci U S A. 2012 Dec 26;109(52):21462-7. doi: 10.1073/pnas.1216830110. Epub 2012 Dec 10.


HDAC6 regulates epidermal growth factor receptor (EGFR) endocytic trafficking and degradation in renal epithelial cells.

Liu W, Fan LX, Zhou X, Sweeney WE Jr, Avner ED, Li X.

PLoS One. 2012;7(11):e49418. doi: 10.1371/journal.pone.0049418. Epub 2012 Nov 13.


Role of genetic modifiers in an orthologous rat model of ARPKD.

O'Meara CC, Hoffman M, Sweeney WE Jr, Tsaih SW, Xiao B, Jacob HJ, Avner ED, Moreno C.

Physiol Genomics. 2012 Aug 1;44(15):741-53. doi: 10.1152/physiolgenomics.00187.2011. Epub 2012 Jun 5.


Transport, cilia, and PKD: must we in (cyst) on interrelationships? Focus on "Increased Na+/H+ exchanger activity on the apical surface of a cilium-deficient cortical collecting duct principal cell model of polycystic kidney disease".

Avner ED, McDonough AA, Sweeney WE Jr.

Am J Physiol Cell Physiol. 2012 May 15;302(10):C1434-5. doi: 10.1152/ajpcell.00070.2012. Epub 2012 Mar 7. No abstract available.


Loss of activator of G-protein signaling 3 impairs renal tubular regeneration following acute kidney injury in rodents.

Regner KR, Nozu K, Lanier SM, Blumer JB, Avner ED, Sweeney WE Jr, Park F.

FASEB J. 2011 Jun;25(6):1844-55. doi: 10.1096/fj.10-169797. Epub 2011 Feb 22.


Diagnosis and management of childhood polycystic kidney disease.

Sweeney WE Jr, Avner ED.

Pediatr Nephrol. 2011 May;26(5):675-92. doi: 10.1007/s00467-010-1656-1. Epub 2010 Oct 29. Review.


Birth of a healthy infant following preimplantation PKHD1 haplotyping for autosomal recessive polycystic kidney disease using multiple displacement amplification.

Lau EC, Janson MM, Roesler MR, Avner ED, Strawn EY, Bick DP.

J Assist Reprod Genet. 2010 Jul;27(7):397-407. doi: 10.1007/s10815-010-9432-5. Epub 2010 May 20.


Polycystic Kidney Disease, Autosomal Recessive.

Sweeney WE, Avner ED.

In: Pagon RA, Adam MP, Ardinger HH, Wallace SE, Amemiya A, Bean LJH, Bird TD, Dolan CR, Fong CT, Smith RJH, Stephens K, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2015.
2001 Jul 19 [updated 2014 Mar 06].


20-HETE activates the Raf/MEK/ERK pathway in renal epithelial cells through an EGFR- and c-Src-dependent mechanism.

Akbulut T, Regner KR, Roman RJ, Avner ED, Falck JR, Park F.

Am J Physiol Renal Physiol. 2009 Sep;297(3):F662-70. doi: 10.1152/ajprenal.00146.2009. Epub 2009 Jul 1.


Chronic blockade of 20-HETE synthesis reduces polycystic kidney disease in an orthologous rat model of ARPKD.

Park F, Sweeney WE Jr, Jia G, Akbulut T, Mueller B, Falck JR, Birudaraju S, Roman RJ, Avner ED.

Am J Physiol Renal Physiol. 2009 Mar;296(3):F575-82. doi: 10.1152/ajprenal.90705.2008. Epub 2009 Jan 7. Erratum in: Am J Physiol Renal Physiol. 2009 Oct;297(4):F1135.


20-HETE mediates proliferation of renal epithelial cells in polycystic kidney disease.

Park F, Sweeney WE, Jia G, Roman RJ, Avner ED.

J Am Soc Nephrol. 2008 Oct;19(10):1929-39. doi: 10.1681/ASN.2007070771. Epub 2008 Jul 2.


Src inhibition ameliorates polycystic kidney disease.

Sweeney WE Jr, von Vigier RO, Frost P, Avner ED.

J Am Soc Nephrol. 2008 Jul;19(7):1331-41. doi: 10.1681/ASN.2007060665. Epub 2008 Apr 2.


Renal cystic disease: new insights for the clinician.

Avner ED, Sweeney WE Jr.

Pediatr Clin North Am. 2006 Oct;53(5):889-909, ix. Review.


Autosomal recessive polycystic kidney disease and congenital hepatic fibrosis: summary statement of a first National Institutes of Health/Office of Rare Diseases conference.

Gunay-Aygun M, Avner ED, Bacallao RL, Choyke PL, Flynn JT, Germino GG, Guay-Woodford L, Harris P, Heller T, Ingelfinger J, Kaskel F, Kleta R, LaRusso NF, Mohan P, Pazour GJ, Shneider BL, Torres VE, Wilson P, Zak C, Zhou J, Gahl WA.

J Pediatr. 2006 Aug;149(2):159-64. No abstract available.


Molecular and cellular pathophysiology of autosomal recessive polycystic kidney disease (ARPKD).

Sweeney WE Jr, Avner ED.

Cell Tissue Res. 2006 Dec;326(3):671-85. Epub 2006 Jun 10. Review.


Haplotype analysis improves molecular diagnostics of autosomal recessive polycystic kidney disease.

Consugar MB, Anderson SA, Rossetti S, Pankratz VS, Ward CJ, Torra R, Coto E, El-Youssef M, Kantarci S, Utsch B, Hildebrandt F, Sweeney WE, Avner ED, Torres VE, Cunningham JM, Harris PC.

Am J Kidney Dis. 2005 Jan;45(1):77-87.

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